(Chest. 2003;124:2388-2390.)
© 2003
American College of Chest Physicians
Respiratory Distress in Woman Who Began Complaining of Dyspnea and Cough 5 Months Ago*
Marvin I. Schwarz, MD, FCCP
* From the ACCP-SEEK program, reprinted with permission. Items are selected by Department Editors Richard S. Irwin, MD, FCCP, and John G. Weg, MD, FCCP. For additional information about the ACCP-SEEK program, phone 1-847-498-1400.
Correspondence to: Marvin I. Schwarz, MD, FCCP, Pulmonary Sciences and Critical Care Medicine, University of Colorado Health Science Center, 4200 East 9th Ave, Box C272, Denver, CO 80262; e-mail: marvin.schwarz{at}uchsc.edu
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Introduction
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A 25-year-old woman without a significant medical history was in excellent health until 5 months ago, when she noted mild dyspnea and cough. She was treated with antibiotics but did not improve. Two months before this hospital admission, her dyspnea became severe, the cough persisted, and she had episodes of hemoptysis. She was hospitalized twice and intubated, treated with diuretics, and discharged. Symptoms persisted, and she is now admitted for further evaluation. Her medications include oxygen, furosemide (40 mg bid), diltiazem (180 mg/d), and warfarin (10 mg/d). Medical, social, family, and occupational histories are noncontributory.
The patient is afebrile and in moderate respiratory distress. Her BP is 102/70 mm Hg, heart rate is 122 beats/min, and respiratory rate is 36 breaths/min. While receiving 3 L/min of nasal oxygen, her oxygen saturation is 91%. There is mild jugular venous distention. Lung auscultation indicates bibasilar crackles. Cardiac examination demonstrates a right-sided third heart sound, a widely split accentuated second heart sound, and a grade 3/6 holosystolic murmur along the left sternal border. Her abdomen is nontender, and the liver is palpated 4 cm below the right costal margin. There is no clubbing or edema.
Arterial blood gas measurements with the patient breathing 3 L/min of nasal oxygen indicate a PO2 of 57 mm Hg, PCO2 of 37 mm Hg, and pH 7.43. The CBC count, sedimentation rate, serum electrolytes, liver tests, and urinalysis are unremarkable. An ECG shows sinus tachycardia. The following serum antibodies are negative: antinuclear cytoplasmic antibody, double-stranded DNA acid, anti-basement membrane antibody, antitoposomerase, and antinuclear factor. An echocardiogram demonstrates normal left ventricular function, right ventricular dilatation with severe tricuspid insufficiency, a mean pulmonary artery pressure of 78 mm Hg, and a normal left atrial size.
A chest radiograph (Fig 1
) was obtained during a prior hospitalization, and a chest radiograph and CT scan (Figs 2
, 3
) are obtained during the current hospitalization. An open-lung biopsy is performed (Fig 4
). All of the following statements about this case are true, EXCEPT?
- A. This condition is usually an idiopathic disorder
- B. The pulmonary artery occlusion pressure is usually normal
- C. Prostacyclin is the recommended treatment
- D. The patient should be evaluated for transplantation
- E. Similar cases occur with the collagen vascular diseases
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Figure 4.. Open-lung biopsy demonstrating two small intralobular septal veins, one of which obliterated by fibrous tissue; the lumen in the other septal vein is narrowed by concentric fibroses.
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Answer: C. Prostacyclin is the recommended treatment
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This patient has pulmonary veno-occlusive disease. The two chest radiographs demonstrate pulmonary hypertension; in addition, Figure 1
shows diffuse alveolar infiltrates due to pulmonary edema or pulmonary hemorrhage resulting from the venous obstruction. The CT scan (Fig 3)
shows ground-glass haze and Kerley B lines, also the result of venous obstruction. The histology demonstrates fibrous obstruction of intralobular septal veins. The major clinical feature is severe pulmonary hypertension; however, plexiform lesions, as seen in familial or spontaneous primary pulmonary hypertension, are not present. Rather, there is narrowing of small pulmonary artery lumens due to medial and intimal hyperplasia, as well as in situ thrombosis and recanalization.
Approximately 6% of patients originally thought to have primary pulmonary hypertension have pulmonary veno-occlusive disease at autopsy. Most cases are idiopathic, but a similar clinicopathologic picture has been reported in rheumatoid arthritis and systemic lupus erythematosus. Cases have been reported following thoracic radiation, as well as chemotherapy with bleomycin and carmustine. It has also been reported in patients with AIDS and sarcoidosis, and following bone marrow transplantation.
Although it would seem that the pulmonary capillary occlusion pressure would be elevated, in practice this is often not the case due to difficulty in positioning, and wedging of the pulmonary artery catheter into unaffected areas. Treatment with IV prostacyclin is not necessarily recommended, as it is in primary pulmonary hypertension, because it has been found to increase pulmonary edema in some cases. Treatment with varying combinations of vasodilators, anticoagulants and corticosteroids, and cytotoxic drugs has been attempted, but most patients succumb within 2 years of diagnosis. Once the diagnosis is made, referral for lung or heart-lung transplantation is recommended.
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Selected Readings
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- Davis, LL, deBoisblanc, BP, Glynn, CE, et al (1995) Effect of prostacyclin on microvascular pressures in a patient with pulmonary veno-occlusive disease. Chest 108,1754-1756[Abstract/Free Full Text]
- Palevsky, HI, Pietra, GG, Fishman, AP Pulmonary veno-occlusive disease and its response to vasodilator agents. Am Rev Respir Dis 1990;142,426-429[ISI][Medline]
- Holcomb, BW, Loyd, JE, Ely, EW, et al Pulmonary veno-occlusive disease: a case series and new observations. Chest 2000;118,1671-1679[Abstract/Free Full Text]
- Mandel, J, Mark, EJ, Hales, CA Pulmonary veno-occlusive disease. Am J Respir Crit Care Med 2000;162,1964-1973[Free Full Text]
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Introduction
Answer: C. Prostacyclin is...
